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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Congenital heart disease has become more prevalent in women of childbearing age and represents about 75% of the heart disease seen in pregnancy. Close monitoring by both obstetricians and cardiologists is advisable for women with complex heart disease, and pregnancy should still be considered contraindicated in several types of congenital heart disease. Women should also be advised of the risk that their offspring may be affected. Women at increased risk for a cardiac event in pregnancy include those with a prior cardiac event or arrhythmia, NYHA functional class > II or cyanosis, left heart obstruction, and systemic ventricular dysfunction. In the absence of adverse predictors, however, women with congenital heart disease can be assured that pregnancy does not pose a significant risk to their health.
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PMID:Congenital heart disease in pregnancy. 1749 72

Congenital heart disease is related to events occurring in the embryonal stage, while rheumatic heart disease is a sequela of immune-mediated damage following streptococcal infection. We report an unusual association of multiple ventricular septal defects and severe pulmonary arterial hypertension with rheumatic mitral stenosis in a 7-year-old girl. This case highlights the need for careful examination for coexisting rheumatic disease in late presentations of congenital heart disease.
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PMID:Juvenile rheumatic mitral stenosis with multiple ventricular septal defects. 1791 Oct 74

Congenital heart disease is associated with arrhythmias. Not only the congenital anatomic component but also the greater arrhythmogenic substrate related to surgical procedures is associated with the increased incidence of arrhythmias in these patients. In pediatric cardiology the study of these phenomena is becoming important and one factor associated with it is the longer follow up, which varies depending on the type of heart disease and arrhythmia. With the same impact, there are daily breakthroughs in diagnostic and treatment through pharmacological and interventional means. The incidence of arrhythmias in post-surgical patients with congenital heart disease is in constant increment, and this is related to the constant increment in the procedures, variety and frequency, as well as the long-term survival.
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PMID:[Incidence and clinical features of arrhythmias after surgical treatment of congenital heart disease]. 1797 79

In 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. This committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease. The working component of this international nomenclature society has been The International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group. The Nomenclature Working Group created the International Paediatric and Congenital Cardiac Code, which is available for free download from the internet at [http://www.IPCCC.NET]. In previous publications from the Nomenclature Working Group, unity has been produced by cross-mapping separate systems for coding, as for example in the treatment of the functionally univentricular heart, hypoplastic left heart syndrome, or congenitally corrected transposition. In this manuscript, we review the nomenclature, definition, and classification of heterotaxy, also known as the heterotaxy syndrome, placing special emphasis on the philosophical approach taken by both the Bostonian school of segmental notation developed from the teachings of Van Praagh, and the European school of sequential segmental analysis. The Nomenclature Working Group offers the following definition for the term "heterotaxy": "Heterotaxy is synonymous with 'visceral heterotaxy' and 'heterotaxy syndrome'. Heterotaxy is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. By convention, heterotaxy does not include patients with either the expected usual or normal arrangement of the internal organs along the left-right axis, also known as 'situs solitus', nor patients with complete mirror-imaged arrangement of the internal organs along the left-right axis also known as 'situs inversus'." "Situs ambiguus is defined as an abnormality in which there are components of situs solitus and situs inversus in the same person. Situs ambiguus, therefore, can be considered to be present when the thoracic and abdominal organs are positioned in such a way with respect to each other as to be not clearly lateralised and thus have neither the usual, or normal, nor the mirror-imaged arrangements."The heterotaxy syndrome as thus defined is typically associated with complex cardiovascular malformations. Proper description of the heart in patients with this syndrome requires complete description of both the cardiac relations and the junctional connections of the cardiac segments, with documentation of the arrangement of the atrial appendages, the ventricular topology, the nature of the unions of the segments across the atrioventricular and the ventriculoarterial junctions, the infundibular morphologies, and the relationships of the arterial trunks in space. The position of the heart in the chest, and the orientation of the cardiac apex, must also be described separately. Particular attention is required for the venoatrial connections, since these are so often abnormal. The malformations within the heart are then analysed and described separately as for any patient with suspected congenital cardiac disease. The relationship and arrangement of the remaining thoraco-abdominal organs, including the spleen, the lungs, and the intestines, also must be described separately, because, although common patterns of association have been identified, there are frequent exceptions to these common patterns. One of the clinically important implications of heterotaxy syndrome is that splenic abnormalities are common. Investigation of any patient with the cardiac findings associated with heterotaxy, therefore, should include analysis of splenic morphology. The less than perfect association between the state of the spleen and the form of heart disease implies that splenic morphology should be investigated in all forms of heterotaxy, regardless of the type of cardiac disease. The splenic morphology should not be used to stratify the form of disease within the heart, and the form of cardiac disease should not be used to stratify the state of the spleen. Intestinal malrotation is another frequently associated lesion that must be considered. Some advocate that all patients with heterotaxy, especially those with isomerism of the right atrial appendages or asplenia syndrome, should have a barium study to evaluate for intestinal malrotation, given the associated potential morbidity. The cardiac anatomy and associated cardiac malformations, as well as the relationship and arrangement of the remaining thoraco-abdominal organs, must be described separately. It is only by utilizing this stepwise and logical progression of analysis that it becomes possible to describe correctly, and to classify properly, patients with heterotaxy.
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PMID:The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. 1803 96

Congenital heart disease is a risk factor for the development of necrotizing enterocolitis, although the exact mechanism of development remains unclear. Herein, we report the case of an infant with pulmonary atresia, an intact ventricular septum, and multiple aortopulmonary collateral vessels. At 4 weeks of age, the infant developed necrotizing enterocolitis in association with significant mesenteric oxygen desaturation, as measured by means of near-infrared spectroscopy. With bowel rest and antibiotic therapy, the patient's mesenteric oxygen saturation and clinical status improved. This case highlights the importance of impaired mesenteric oxygen delivery consequential to congenital heart disease as a possible risk factor for necrotizing enterocolitis, and the use of near-infrared spectroscopy to measure tissue perfusion noninvasively in high-risk patients. To our knowledge, this is the 1st report of mesenteric oxyhemoglobin desaturation in association with necrotizing enterocolitis in a patient who also had congenital heart disease.
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PMID:Mesenteric oxygen desaturation in an infant with congenital heart disease and necrotizing enterocolitis. 1817 26

The prevalence of migraine headaches (MH) is 12% in the general population and increases to 40% in patients with patent foramen ovale. This study evaluated the prevalence of MH in patients with congenital heart disease (CHD). Of 466 patients contacted from the UCLA Adult Congenital Heart Disease Center, 395 (85%) completed a questionnaire to determine the prevalence of MH. Patients were stratified by diagnosis of right-to-left, left-to-right, or no shunt. A group of 252 sex-matched patients with acquired cardiovascular disease served as controls. The prevalence of MH was 45% in adults with CHD compared to 11% in the controls (p<0.001). Of the 179 patients with MH, 143 (80%) had migraines with aura and 36 (20%) had migraines without aura versus 36% and 64% observed in the controls (p<0.001). The frequency of MH was 52% in the right-to-left shunt group, 44% in the left-to-right, and 38% in the no shunt group (p=NS). In patients with a right-to-left shunt who underwent surgical repair, 47% had complete resolution of MH, whereas 76% experienced >50% reduction in headache days per month. In conclusion, the prevalence of MH in all groups of adults with CHD is 3 to 4 times more than a sex-matched control population, with increasing prevalence of MH in patients with no shunt, left-to-right, and right-to-left shunt. The higher than expected frequency of MH in patients with CHD without an intracardiac shunt, suggests additional mechanisms to explain the significant association with MH.
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PMID:Prevalence of migraine headaches in patients with congenital heart disease. 1823 8

Congenital heart disease is associated with arrhythmias. Not only the congenital anatomic settings but also the arrhythmogenic substrate related to surgical procedures is associated with the increased incidence of arrhythmias in these patients. In the National Institute of Cardiology, we reviewed retrospectively 128 clinical records of patients subjeted to surgical treatment of congenital heart disease (81 Fontan surgery, 19 with anomalous pulmonary drainage, 19 Fallot tetralogy). The incidence of supraventricular tachycardia was 8.6%, and the bradyarrhythmias were 15% with AV block in different degrees. Permanent pacing was required in 7.8% of the patients. The incidence of arrhythmias in post-surgical patients with congenital heart disease is increasing constantly.
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PMID:[Incidence and clinical behavior of arrhythmias associated with congenital heart disease and surgical treatment]. 1836 Oct 74

Congenital heart disease is the commonest malformation in humans and contributes greatly to the burden of disease in infancy. Increasingly, developmental origins are also implicated in heart disease in adults. Significant advances have been made over the past decade in elucidating morphogenetic events of heart formation and their underlying molecular cascades, mostly in animal models. Clinical studies are increasingly successful in quantifying and unraveling genetic factors. This review focuses on recent progress made in understanding the genetic underpinnings of normal and abnormal heart formation and highlights the importance of understanding these mechanisms to improve patient management.
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PMID:Genetic factors in congenital heart malformation. 1846 50

To estimate incidence of cleft lip and cleft palate among Jordanian infants and to identify associated anomalies, we carried out a prospective, hospital-based study over a 5-year period, January 2000-January 2005. The total number of liveborn infants was 25 440, 60 of whom (2.4/1000) had facial clefts: 20 (33%) with cleft lip, 15 (25%) with cleft palate and 25 (42%) with both. Congenital heart disease was the commonest associated anomaly (47%) identified, followed by skeletal abnormalities (13%). Renal anomalies were found in 10% of cases. The high prevalence of congenital heart disease may justify routine echocardiography screening. The high consanguineous marriage rate may be a contributing factor to the high incidence of cleft anomalies.
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PMID:Facial cleft and associated anomalies: incidence among infants at a Jordanian medical centre. 1856 27

Congenital heart disease is the most prevalent type of inborn defect, with a rapidly growing and aging patient population that is increasing in complexity. Indicative of the remarkable progress in pediatric cardiac care, adults now outnumber children with congenital heart disease. Adults with congenital heart disease constitute a distinct and heterogeneous population of patients with unique needs, concerns, and challenges. Arrhythmias figure foremost among the issues encountered and are the leading cause of morbidity, hospital admissions, and mortality. Several novel and singular challenges of unparalleled diversity are encountered in the arrhythmia management of adults with congenital heart disease and span the entire spectrum of bradyarrhythmias and tachyarrhythmias. The nascent field of adult congenital electrophysiology requires an integration of standard electrophysiology proficiencies with a thorough appreciation for congenital heart disease anatomy, physiology, and surgical interventions. The objective of this review is to highlight commonly confronted arrhythmia issues and themes, discuss particular challenges, review recent relevant literature, and summarize current management trends.
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PMID:EP challenges in adult congenital heart disease. 1918 1


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